Autoimmune hemolytic anemia as a complication of congenital anemias. A case series and review of the literature

Irene Motta, Juri Giannotta, Marta Ferraresi, Kordelia Barbullushi, Nicoletta Revelli, Giovanna Graziadei, Wilma Barcellini, Bruno Fattizzo

Research output: Contribution to journalArticlepeer-review

Abstract

Congenital anemias may be complicated by immune-mediated hemolytic crisis. Alloantibodies are usually seen in chronically transfused patients, and autoantibodies have also been described, although they are rarely associated with overt autoimmune hemolytic anemia (AIHA), a serious and potentially life-threatening complication. Given the lack of data on the AIHA diagnosis and management in congenital anemias, we retrospectively evaluated all clinically relevant AIHA cases occurring at a referral center for AIHA, hemoglobinopathies, and chronic hemolytic anemias, focusing on clinical management and outcome. In our cohort, AIHA had a prevalence of 1% (14/1410 patients). The majority were warm AIHA. Possible triggers were recent transfusion, infection, pregnancy, and surgery. All the patients received steroid therapy as the first line, and about 25% required further treatment, including rituximab, azathioprine, intravenous immunoglobulins, and cyclophosphamide. Transfusion support was required in 57% of the patients with non-transfusion-dependent anemia, and recombinant human erythropoietin was safely administered in one third of the patients. AIHA in congenital anemias may be challenging both from a diagnostic and a therapeutic point of view. A proper evaluation of hemolytic markers, bone marrow compensation, and assessment of the direct antiglobulin test is mandatory.

Original languageEnglish
Article number3439
JournalJournal of Clinical Medicine
Volume10
Issue number15
DOIs
Publication statusPublished - Aug 1 2021

Keywords

  • Alloimmunization
  • Autoimmune hemolytic anemia
  • Congenital hemolytic anemias
  • Sickle cell disease
  • Thalassemia

ASJC Scopus subject areas

  • Medicine(all)

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